Chylous Manifestations and Management of Gorham-Stout Syndrome
Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presen...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Korean Society for Thoracic and Cardiovascular Surgery
2019-02-01
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| Series: | Korean Journal of Thoracic and Cardiovascular Surgery |
| Subjects: |
| _version_ | 1819084515152232448 |
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| author | Sungbin Cho Seung Ri Kang Beom Hee Lee Sehoon Choi |
| author_facet | Sungbin Cho Seung Ri Kang Beom Hee Lee Sehoon Choi |
| author_sort | Sungbin Cho |
| collection | DOAJ |
| description | Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus
(0.8 mg/m2, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years. |
| first_indexed | 2024-12-21T20:49:41Z |
| format | Article |
| id | doaj.art-369c0e43f0874816ace2d1806498b433 |
| institution | Directory Open Access Journal |
| issn | 2233-601X 2093-6516 |
| language | English |
| last_indexed | 2024-12-21T20:49:41Z |
| publishDate | 2019-02-01 |
| publisher | Korean Society for Thoracic and Cardiovascular Surgery |
| record_format | Article |
| series | Korean Journal of Thoracic and Cardiovascular Surgery |
| spelling | doaj.art-369c0e43f0874816ace2d1806498b4332022-12-21T18:50:44ZengKorean Society for Thoracic and Cardiovascular SurgeryKorean Journal of Thoracic and Cardiovascular Surgery2233-601X2093-65162019-02-01521444610.5090/kjtcs.2019.52.1.44Chylous Manifestations and Management of Gorham-Stout SyndromeSungbin Cho0Seung Ri Kang1Beom Hee Lee2Sehoon Choi3Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of MedicineDepartment of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of MedicineDepartment of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of MedicineDepartment of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of MedicineGorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus (0.8 mg/m2, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years.Gorham-Stout diseasePleural effusionSirolimusChylothorax |
| spellingShingle | Sungbin Cho Seung Ri Kang Beom Hee Lee Sehoon Choi Chylous Manifestations and Management of Gorham-Stout Syndrome Korean Journal of Thoracic and Cardiovascular Surgery Gorham-Stout disease Pleural effusion Sirolimus Chylothorax |
| title | Chylous Manifestations and Management of Gorham-Stout Syndrome |
| title_full | Chylous Manifestations and Management of Gorham-Stout Syndrome |
| title_fullStr | Chylous Manifestations and Management of Gorham-Stout Syndrome |
| title_full_unstemmed | Chylous Manifestations and Management of Gorham-Stout Syndrome |
| title_short | Chylous Manifestations and Management of Gorham-Stout Syndrome |
| title_sort | chylous manifestations and management of gorham stout syndrome |
| topic | Gorham-Stout disease Pleural effusion Sirolimus Chylothorax |
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